Systemic Lupus Erythmatosus
Definition Systemic Inflammatory autoimmune disorder affecting multiple organ systems characterized by the roduction of autoantibodies directed against cell nuclei Epidemiology Nine times more common in Woemn vs Men. Peak age of onset between 20-40 years. Etiology Combination of Genetic, Immunological (HLA genes, Complement Deficiency, ?EBV link), Endocrine (Premenopausal, flares from HRT) and Environmental (UV light) interactions. Drugs Withdraw drug. High risk. Procainamide and Hydralazine Low Risk *Antimicrobials. Isoniazid, Minocycline, Pyrazinamide. *Anti-convulsants. Carbamezepine, Oxacarbazepine, Phenytoin *Others. TNF-α Inhibitors Infliximab, Etanercept. Antiarrythmics Quinidine, Propafenone. Anti-inflammatory such as D-Penicillamine. Clinical Features Constitutional : Fever, Fatigue, Weight loss Mucocutaneous (~85%) : Malar, discoid, maculopapular, rash. pannicculitis, vasculitis (small vessel, Raynaud's Phenomenon, livedo reticularis, purpura, alopecia, oral ulcers Musculoskeletal : Nonerosive, trainseint. Symmetrical, small joints. Rarely deforming (Jaccoud's Arthopathy). Similar to RA but less severe. Synovitis. Osteoporosis, Avascular necrosis (both multifactorial), Myalgia, Myositis. Renal : Lupus nephritis in upto 50%. 10% go to dialysis or transplant Pulmonary : Pleurisy, effusion (Exudative) Pneumonitis, Pulmnary hemorrhage, Shriking lung, Cardiac : Pericarditis, pericardial effusion (beware tamponade), myocarditis, endocaridtis (Aortic > Mitral). CAD Gastro-intestinal : Vasculitis of gut. Diarrhea, abdominal pain. Hepatic abnormalities from treatment. PUD from NSAIDs Hematological : Autoimmune hemolyic anemia (AIHA, MAHA) Leukopenia, Lymphopenia, Thrombocytopenia Ocular : Retinal vasculitis. Cotton wool spots. Ocular invovlement correlates to active systemic disease. Secondary Sjögren's Syndrome Central Nervous System : Neuropsychiatric Complications. Wide array including mood disorders, anxiety, psychosis, cognition. Movement disorder, Seizures, Neuropathy (including autonomic) Diagnostic Criteria 4 out of 11. Simultaneously or not, overy any time period. Clinical Features Skin and Mucosa *Malar rash *Discoid Rash *Photosensitivity *Painless oral ulcers Joints : ''Non erosive arthritis in 2 or more pheripheral joints ''Mesothelium : ''Pericarditis or pleuritis. Evidence by rubs, ECG, effusions ''Neurological : ''Seizures or psychosis in absence of other causes such as metabolic derangements. Laboratory Features ''Renal : Perisitent proteinuria >0.5g/day, >3+ on dipstick, OR cellular casts Hematologic : Hemolytic anemia (With reticulocytosis) OR Leukopenia (< 4000) OR Lymphopenia (<1,5000) on more than 3 occasions. OR Thrombocytopenia < 100.0000 in absence of offending drugs Immunologic : *Anti-dsDNA *Anti-Sm *Anti-phospholipid antibodies - anti-cardiolipin, +ve lupsus anticoagulant, false positive T pallidum confirmed by further antibody testing) Positive ANA in absence of drugs Investigations Blood Tests *Full blood count looking for leukopenia, lymphopenia, thrombocytopenia. Anemia of chronic disease or autoimmune hemolytic anemia. *ESR proportional to disease activity *CRP may be raised in infection or pleuritis, arthritis *Renal Panel. Raised urea and creatinine in renal involvement. *Antibodies especially anti-dsDNA, anti-Sm and ANA *Serum complement. C3, C4 reduced in active disease Imaging *Chest x-ray looking for pleural effusions or pericardial effusions *Ultrasound looking for valvular involvement *CT abdomen in gut involvement *CT/MRI brain looking for infarcts, hemorrhage. Histology *Renal, skin biopsies show IgG and Complement Deposition Managment Proper counselling of the patient regarding chronicity, complications of disease and of treatment Lifestyle *Photosensitivity - Avoid sun, use sunblock *Hygiene *Exercise and Diet in view of increased CVS risk *Contraception Pharmacotherapy Mild SLE *Antimalarials eg Hydroxychloroquine. Cutaneous, musculoskeletal, cardioprotective against atherosclerosis. *Low dose glucocorticoids Moderate-severe SLE *Corticosteroids eg methylprednisolone, hydrocortisone, prednisolone *Immunosuppressants eg Azathioprine, Mycophenolate Mofetil, Cyclophosphamide, Rituximab (monoclonal Anti-CD20) Surgical For complications of SLE such as Renal Failure, Valvulopathy, Bowel infarction. Preventive Care Smoking cessation Yearly Influenza vaccination Pneumococcal vaccination 5 yearly Preventive cancer screening Prognosis Chronic disease. Episodic flares. 10 year survival ~90% Worse prognosis if major organ invovlement Leading causes of death *Active Lupus *Infection *CVS disease *Cerebral disease *Lupus Nephritis Lupus and Pregnancy Multidisciplinary management with Rheumatologist, Obstetrician experienced in high risk pregnancies and nephrologist if kindey invovlement. From Medscape: SLE increases risk of *spontaneous abortion *intrauterine fetal death *preecamplsia *intrauterine growth retardation *preterm birth *Neonatal lupus Best prognosis *disease quiescent for six months or more. *normal, or very mild renal involvement Category:Internal Medicine Category:Rheumatology